Lysosomal acid lipase deficiency causes respiratory inflammation and destruction in the lung

American Journal of Physiology - Lung Cellular and Molecular Physiology

Volumn 286, Issue 4 30-4, 2004, Pages

  Lian, Xuemei ^a,b   Yan, Cong ^a   Yang, Li ^a   Xu, Yan ^a   Du, Hong ^a  

^a CINCINNATI CHILDREN'S HOSPITAL MEDICAL CENTER   (United States)

^b CHONGQING MEDICAL UNIVERSITY   (China)

Author keywords

Emphysema; Neutral lipid; Remodeling

Indexed keywords

ACID LIPASE; CHOLESTEROL ESTER; CYTOKINE; GELATINASE B; INTERLEUKIN 1BETA; INTERLEUKIN 6; LIPID; MACROPHAGE ELASTASE; MESSENGER RNA; NEUTROPHIL COLLAGENASE; TRIACYLGLYCEROL; TUMOR NECROSIS FACTOR ALPHA;

ANIMAL TISSUE; ARTICLE; CELL HYPERPLASIA; CELL PROLIFERATION; CLARA CELL; CONTROLLED STUDY; DISEASE COURSE; DISEASE SEVERITY; HOMEOSTASIS; INFLAMMATION; LUNG ALVEOLUS MACROPHAGE; LUNG INJURY; LYSOSOME; METABOLISM; MOUSE; NEUTROPHIL; NONHUMAN; PATHOPHYSIOLOGY; PHENOTYPE; PRIORITY JOURNAL; PROTEIN EXPRESSION; WOLMAN DISEASE;

EID: 1642379630     PISSN: 10400605     EISSN: None     Source Type: Journal    
DOI: 10.1152/ajplung.00335.2003     Document Type: Article

References (35)

1. Atkinson JJ and Senior RM. Matrix metalloproteinase-9 in lung remodeling. Am J Respir Cell Mol Biol 28: 12-24, 2003.
2. Chen H, Lin RJ, Schiltz RL, Chakravarti D, Nash A, Nagy L, Privalsky ML, Nakatani Y, and Evans RM. Nuclear receptor coactivator ACTR is a novel histone acetyltransferase and forms a multimeric activation complex with P/CAF and CBP/p300. Cell 90: 569-580, 1997.
3. Du H, Duanmu M, Witte D, and Grabowski GA. Targeted disruption of the mouse lysosomal acid lipase gene: long-term survival with massive cholesteryl ester and triglyceride storage. Hum Mol Genet 7: 1347-1354, 1998.
4. a. Du H, Heur M, Duanmu M, Grabowski GA, Hui DY, Witte DP, and Mishra J. Lysosomal acid lipase-deficient mice: depletion of white and brown fat, severe hepatosplenomegaly, and shortened life span. J Lipid Res 42: 489-500, 2001.
5. Du H, Heur M, Witte DP, Ameis D, and Grabowski GA. Lysosomal acid lipase deficiency: correction of lipid storage by adenovirus-mediated gene transfer in mice. Hum Gene Ther 13: 1361-1372, 2002.
6. Du H, Schiavi S, Levine M, Mishra J, Heur M, and Grabowski GA. Enzyme therapy for lysosomal acid lipase deficiency in the mouse. Hum Mol Genet 10: 1639-1648, 2001.
7. 2 is a ligand for the adipocyte determination factor PPAR-γ. Cell 83: 803-812, 1995.
8. Harrod KS, Mounday AD, Stripp BR, and Whitsett JA. Clara cell secretory protein decreases lung inflammation after acute virus infection. Am J Physiol Lung Cell Mol Physiol 275: L924-L930, 1998.
9. Jiang C, Ting AT, and Seed B. PPAR-γ agonists inhibit production of monocyte inflammatory cytokines. Nature 391: 82-86, 1998.
10. Kamei Y, Xu L, Heinzel T, Torchia J, Kurokawa R, Gloss B, Lin SC, Heyman RA, Rose DW, Glass CK, and Rosenfeld MG. A CBP integrator complex mediates transcriptional activation and AP-1 inhibition by nuclear receptors. Cell 85: 403-414, 1996.
11. 2 induces synoviocyte apoptosis and suppresses adjuvant-induced arthritis in rats. J Clin Invest 106: 189-197, 2000.
12. Kraal G, van der Laan LJ, Elomaa O, and Tryggvason K. The macrophage receptor MARCO. Microbes Infect 2: 313-316, 2000.
13. Lee WL and Downey GP. Neutrophil activation and acute lung injury. Curr Opin Crit Care 7: 1-7, 2001.
14. Moraes TJ, Chow CW, and Downey GP. Proteases and lung injury. Crit Care Med 31: S189-S194, 2003.
15. Mukaida N. Pathophysiological roles of interleukin-8/CXCL8 in pulmonary diseases. Am J Physiol Lung Cell Mol Physiol 284: L566-L577, 2003.
16. Nagy L, Tontonoz P, Alvarez JG, Chen H, and Evans RM. Oxidized LDL regulates macrophage gene expression through ligand activation of PPARγ. Cell 93: 229-240, 1998.
17. Naltner A, Wert S, Whitsett JA, and Yan C. Temporal/spatial expression of nuclear receptor coactivators in the mouse lung. Am J Physiol Lung Cell Mol Physiol 279: L1066-L1074, 2000.
18. Onate SA, Tsai SY, Tsai MJ, and O'Malley BW. Sequence and characterization of a coactivator for the steroid hormone receptor superfamily. Science 270: 1354-1357, 1995.
19. Ricote M, Li AC, Willson TM, Kelly CJ, and Glass CK. The peroxisome proliferator-activated receptor-γ is a negative regulator of macrophage activation. Nature 391: 79-82, 1998.
20. Sallenave JM and Ryle AP. Purification and characterization of elastase-specific inhibitor. Sequence homology with mucus proteinase inhibitor. Biol Chem Hoppe Seyler 372: 13-21, 1991.
21. Sallenave JM, Shulmann J, Crossley J, Jordana M, and Gauldie J. Regulation of secretory leukocyte proteinase inhibitor (SLPI) and elastase-specific inhibitor (ESI/elafin) in human airway epithelial cells by cytokines and neutrophilic enzymes. Am J Respir Cell Mol Biol 11: 733-741, 1994.
22. Sallenave JM, Silva A, Marsden ME, and Ryle AP. Secretion of mucus proteinase inhibitor and elafin by Clara cell and type II pneumocyte cell lines. Am J Respir Cell Mol Biol 8: 126-133, 1993.
23. Shapiro SD and Senior RM. Matrix metalloproteinases. Matrix degradation and more. Am J Respir Cell Mol Biol 20: 1100-1102, 1999.
24. Shipley JM, Wesselschmidt RL, Kobayashi DK, Ley TJ, and Shapiro SD. Metalloelastase is required for macrophage-mediated proteolysis and matrix invasion in mice. Proc Natl Acad Sci USA 93: 3942-3946, 1996.
25. Singh G and Katyal SL. Clara cell proteins. Ann NY Acad Sci 923: 43-58, 2000.
26. Su CG, Wen X, Bailey ST, Jiang W, Rangwala SM, Keilbaugh SA, Flanigan A, Murthy S, Lazar MA, and Wu GD. A novel therapy for colitis utilizing PPAR-γ ligands to inhibit the epithelial inflammatory response. J Clin Invest 104: 383-389, 1999.
27. Torchia J, Rose DW, Inostroza J, Kamei Y, Westin S, Glass CK, and Rosenfeld MG. The transcriptional co-activator p/CIP binds CBP and mediates nuclear-receptor function. Nature 387: 677-684, 1997.
28. Trapnell BC and Whitsett JA. GM-CSF regulates pulmonary surfactant homeostasis and alveolar macrophage-mediated innate host defense. Annu Rev Physiol 64: 775-802, 2002.
29. Visse R and Nagase H. Matrix metalloproteinases and tissue inhibitors of metalloproteinases-structure, function and biochemistry. Circ Res 92: 827-839, 2003.
30. Voegel JJ, Heine MJ, Zechel C, Chambon P, and Gronemeyer H. TIF2, a 160-kDa transcriptional mediator for the ligand-dependent activation function AF-2 of nuclear receptors. EMBO J 15: 3667-3675, 1996.
31. Wang AC, Dai X, Luu B, and Conrad DJ. Peroxisome proliferator-activated receptor-γ regulates airway epithelial cell activation. Am J Respir Cell Mol Biol 24: 688-693, 2001.
32. Winyard PG. Key stages in the acute inflammatory response and their relevance as therapeutic targets. Methods Mol Biol 225: 3-6, 2003.
33. Xu Y, Clark JC, Aronow BJ, Dey CR, Liu C, Wooldridge JL, and Whitsett JA. Transcriptional adaptation to cystic fibrosis transmembrane conductance regulator deficiency. J Biol Chem 278: 7674-7682, 2003.
34. Yang L, Naltner A, and Yan C. Overexpression of dominant negative retinoic acid receptor-α causes alveolar abnormality in transgenic neonatal lungs. Endocrinology 144: 3004-3011, 2003.
35. Yang L, Yan D, Yan C, and Du H. PPARγ and ligands inhibit SP-B gene expression in the lung. J Biol Chem 278: 36841-36847, 2003.